Niños y adolescentes con enfermedades raras atendidos en consulta provincial de nutrición / Children and Adolescents with Rare Diseases Treated in Provincial Nutrition Consultation

RESUMEN Fundamento: el padecimiento de una enfermedad rara produce dificultades para su diagnóstico oportuno. Su pronóstico ensombrece cuando se asocia a desnutrición. Objetivo: caracterizar a niños y adolescentes con enfermedades raras y desnutrición, atendidos en consulta provincial de nutrición en Cienfuegos. Métodos: se realizó un estudio descriptivo, retrospectivo y transversal cuyo universo lo constituyeron los 13 niños y adolescentes con enfermedades raras y desnutrición de gravedad variable que acudieron ese periodo a consulta en el Hospital Pediátrico de Cienfuegos en el entre enero y junio del 2017. Los datos fueron obtenidos de la revisión de la historia clínica, la entrevista médica, valoración clínica y nutricional. Las variables analizadas fueron: edad, sexo, tipo de desnutrición, peso al nacer, hábitos alimentarios y alteraciones en aparatos y sistemas. Resultados: hubo predominio del sexo masculino con 9 (69,2 %) y del grupo mayor de 15 años, con 5 (38,4 %). La desnutrición moderada fue la que más se manifestó con 6 (46,1 %). Es representativo que 8 pacientes exhibieron un peso al nacer de más de 3 000 gramos, para un 61,5 %, mientras que los hábitos alimentarios adecuados se presentaron con más frecuencia, resaltan los pacientes evaluados como desnutridos moderados dentro del grupo. El 100 % de los pacientes presentan alteración musculo-esquelética. La baja talla fue la más frecuente de las afectaciones del crecimiento y desarrollo, con un 46,1 %. Los pacientes con las formas severas de desnutrición presentan una mayor expresividad de la sintomatología. Conclusión: predominó como el más afectado, el grupo de más de 15 años, el sexo masculino, el peso normal al nacer y los hábitos alimentarios adecuados. Fueron las alteraciones musculo-esqueléticas representativas en todos los casos.

ABSTRACT Foundation: suffering an uncommon disease causes difficulties for its timely diagnosis. Their prognosis overshadows when associated with malnutrition. Objective: to characterize children and adolescents with uncommon diseases and malnutrition, assisted in provincial nutritional consultation in Cienfuegos. Methods: a descriptive, retrospective and cross-sectional study whose universe consisted of 13 children and adolescents with uncommon diseases and malnutrition of variable severity who attended the Pediatric Hospital consultation of Cienfuegos. Data were obtained from clinical history review, medical interview and clinical and nutritional assessment. The following variables were analyzed: age, sex, type of malnutrition, birth weight, eating habits and alterations in apparatus and systems. The results were taken to a database for statistical processing by the SPSS program version 15,0. The results were presented in frequency and ratio tables of variables expressed in frequency number and in percentages. Results: there was predominance of the male sex with 9 (69,2 %) and of the group over 15 years old, with 5 (38,4 %). Moderate malnutrition was the frequent with 6 (46,1 %). It is representative that 8 patients exhibited a birth weight of more than 3,000 grams, for 61,5 %, while adequate eating habits occurred more frequently, highlighting the patients evaluated as moderate malnourished within the group. All of the patients have musculoskeletal disorders. The short stature was the most frequent of growth and development affectations, with 46,1 %. Patients with severe forms of malnutrition present a greater symptomatology expressivity. Conclusion: the most affected group was male sex over 15 years old, the normal weight at birth and the adequate eating habits prevailed. There were the representative musculoskeletal alterations in all cases.

Phytoestrogen treatment induces testis alterations in dogs. Potential use in population control.

Dog overpopulation is considered a human health risk; they are the terrestrial vector of rabies and reservoirs for other human diseases. Surgical neutering and intratesticular injections have been used in male dogs. Physiological and morphological alterations in reproductive organs can be induced by phytoestrogens. Our goal was to evaluate the effect of oral coumestrol on dog ejaculates and testis histology. Two groups of 5 healthy adult dogs were used. One coumestrolcontaining biscuit was given once a week for a 4 week period to the experimental group. Ejaculates were obtained and evaluated. After treatment, testis were obtained and processed for histology. Compared to controls, treated dogs have reduced tubules (462 +/- 1.4 vs 336 +/- 2 micron(2)), spermatogenic epithelium (49.1 +/- 0.01 vs 13.3 +/- 0.01 micron(2)), and lumen opening (891 +/- 1.4 vs 530 +/- 26.9 micron). Ejaculates from treated animals have increased numbers of abnormal spermatozoa and reduced sperm concentration.

Fluorescence and inmunohistological detection of estrogen receptors in dog testis and epidydimis after oral coumestrol administration.

INTRODUCTION: Estrogens are well recognized as important hormones in male reproduction and act as ligands to alpha and beta estrogen receptors. Both estrogen receptors could interact with estrogen-mimicking compounds such as the fluorescent phytoestrogen coumestrol, which acts both in an agonist or antagonist fashion. OBJECTIVE: To investigate the presence of Coumestrol-Estrogen Receptor complexes by fluorescence in testis and epididymis, its effect in the ER expression by immunostain in the same tissues and the effect of this binding in the testis histological characteristics. DESIGN: Adult healthy and sexually active dogs were assigned to either the experimental or control group .Coumestrol impregnated dog biscuits were given to each animal from the experimental group once a week for a 4 week period. The control group received a biscuit with no Coumestrol, also once a week and for the same period. Testis morphology, ER immunodetection, and coumestrol-receptor binding were evaluated. SETTING: The experiment was done in the facilities of the Mexico City canine shelter. Animals were caged individually with food and water ad libitum and having at least two daily hours for exercise. RESULTS: Morphological alterations in testis after oral administration of coumestrol were detected. The main alterations include decreased germinal epithelium in tubule, and the loss of a continuous proliferation and differentiation gamete layer. Fluorescence signals in testis interstitial Leydig cells and epididymus indicating ER-coumestrol complexes were detected at the same points to those Immunohystochemically detected ER. CONCLUSIONS: Coumestrol administration induces testis alterations and coumestrol-ER complexes can be co-localized by binding-enhanced fluorescence and immunoprecipitation.